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Sun-induced disorders.

Photosensitivity diseases frequently occur as a result of sun exposure in individuals with inherited and acquired disorders. Several of these disorders may manifest acute cutaneous manifestations that bring the patient to the emergency room. The more common disorders that may be seen in this setting include sunburn, lupus erythematosus, porphyria, photosensitivity dermatitis, and polymorphous light eruption. The diagnosis can frequently be suspected on the basis of a careful history and physical examination. Specialized diagnostic procedures available to the dermatologist may assist in making the correct diagnosis. Effective treatment is available for many of these disorders.

Adult↗

Results of evaluation of 203 patients for photosensitivity in a 7.3-year period.

BACKGROUND: Although photosensitivity disorders have been well described, their incidence in a referral institution had not been studied. OBJECTIVE: The purpose of this study was to evaluate the incidence of photosensitivity disorders, including photocontact dermatitis, in an academic medical center. METHODS: The results of the assessment of 203 consecutive patients, all of whom had phototests, referred for the evaluation of photosensitivity disorders during a 7.3-year period were reviewed. RESULTS: The mean age was 50 years, and 63% of the patients were women. The most frequent diagnoses were polymorphous light eruption (26% of the total patient population), chronic actinic dermatitis (17%), photoallergic contact dermatitis (8%), systemic phototoxicity to therapeutic agents (7%), and solar urticaria (4%). Positive photopatch reactions, patch test reactions, or both were observed in 40 (29%) of the 138 tested patients. The frequencies of the positive photopatch test reactions were sunscreens (57%), fragrances (18%), and antimicrobial agents (13%). Of the positive patch test responses, fragrances elicited 47% of the total positive reactions, followed by sunscreens (39%) and antimicrobial agents (7%). CONCLUSION: Polymorphous light eruption, chronic actinic dermatitis, and photoallergic contact dermatitis were the most frequently made diagnoses. Sunscreens, fragrances, and antimicrobial agents were the most common clinically relevant photoallergens and allergens.

Academic Medical Centers↗

An overview of equine dermatoses characterized by scaling and crusting.

Scaling and/or crusting are common clinical findings associated with equine skin diseases. Scaling and crusting may be associated with pruritic or nonpruritic dermatoses. This article focuses on those conditions that are usually nonpruritic in horses. They include the infectious dermatoses, the keratinization/seborrheic disorders, photosensitization, and the immunologic/inflammatory disorders, including pemphigus foliaceus, equine exfoliative eosinophilic dermatitis and stomatitis, and equine histiocytic dermatitis (sarcoidosis). Clinical signs that help differentiate the various disorders are given, and diagnostic and therapeutic options are discussed.

Animals↗

Artificial light sources.

A wide variety of artificial light sources exists for use in the diagnosis and treatment of photosensitivity disorders. A discussion of the advantages and disadvantages of these light sources (including gas discharge arcs, fluorescent lamps, and other apparatus) illustrates the importance of matching the emission spectrum of the light source, the spectral response of the radiometer, and the photobiologic action spectrum. Environmental and occupational exposure to artificial light sources may contribute to photosensitivity disorders.

Carbon↗

Complete absence of Cockayne syndrome group B gene product gives rise to UV-sensitive syndrome but not Cockayne syndrome.

UV-sensitive syndrome (UVsS) is a rare autosomal recessive disorder characterized by photosensitivity and mild freckling but without neurological abnormalities or skin tumors. UVsS cells show UV hypersensitivity and defective transcription-coupled DNA repair of UV damage. It was suggested that UVsS does not belong to any complementation groups of known photosensitive disorders such as xeroderma pigmentosum and Cockayne syndrome (CS). To identify the gene responsible for UVsS, we performed a microcell-mediated chromosome transfer based on the functional complementation of UV hypersensitivity. We found that one of the UVsS cell lines, UVs1KO, acquired UV resistance when human chromosome 10 was transferred. Because the gene responsible for CS group B (CSB), which involves neurological abnormalities and photosensitivity as well as a defect in transcription-coupled DNA repair of UV damage, is located on chromosome 10, we sequenced the CSB gene from UVs1KO and detected a homozygous null mutation. Our results indicate that previous complementation analysis of UVs1KO was erroneous. This finding was surprising because a null mutation of the CSB gene would be expected to result in CS features such as severe developmental and neurological abnormalities. On the other hand, no mutation in the CSB cDNA and a normal amount of CSB protein was detected in Kps3, a UVsS cell line obtained from an unrelated patient, indicating genetic heterogeneity in UVsS. Possible explanations for the discrepancy in the genotype-phenotype relationship in UVs1KO are presented.

Base Sequence↗

Spectrum of idiopathic photodermatoses in a Mediterranean country.

BACKGROUND: Idiopathic photodermatoses are considered to be common disorders in the population of northern latitude countries, presumably because of the dominance of more "sun-sensitive" individuals with a light-skinned complexion. The incidence of these disorders in the Mediterranean or tropical countries is often under-appreciated because of the higher degree of perennial presence of sunlight and the prevalence of darker skin-type individuals who are seemingly more resistant to the development of sun sensitivity. METHODS: We performed a retrospective, chart-based review of all patients who were diagnosed with idiopathic photodermatoses at a photodermatology referral center in Athens, Greece, during a period of 10 years. Our aim was to assess the pattern of idiopathic photosensitivity disorders in a Mediterranean country and to determine their epidemiological, clinical, and photobiological profile. RESULTS: A total of 310 patients were referred to our center with symptoms of photosensitivity. One hundred and forty-six patients (47.0%) were diagnosed with an idiopathic photosensitivity disorder by means of history, clinical examination, biochemical screening, histology, and phototesting. The most prevalent disorder was polymorphous light eruption, which was diagnosed in 95 patients (65.0%) of our cohort. Chronic actinic dermatitis occurred in 15 patients (10.2%), solar urticaria in 26 patients (17.8%), actinic prurigo in three patients (2.0%), hydroa vacciniforme in one patient (0.6%) and juvenile spring eruption in six patients (4.1%). CONCLUSIONS: Compared with the results of other studies, the prevalence of idiopathic photodermatoses appears to have a similar trend to that of higher latitude countries. Distinct features in our series include the higher incidence of idiopathic photosensitivity in patients with a fair-skinned complexion (skin types II-III) and the frequent appearance of photo-induced eruptions during sunny weather breaks in the winter period.

Adolescent↗

Protection against photodynamic therapy (PDT)-induced photosensitivity by fabric materials.

"Special" highly protective fabrics are now available that offer broad-spectrum protection in preventing sunburn, and possibly other types of photodamage. It is important to know to what extent these fabrics are capable of protecting the wearer against skin cancer, photosensitivity disorders, and inadvertent phototoxic reactions from photodynamic therapy (PDT). We assess the ability of one such special (Solumbra) fabric and one "typical" summer fabric to provide protection against PDT phototoxicity produced in tape-stripped Sk-1 hairless mice by topical 5-aminolevulinic acid (ALA) and (primarily) visible light (360-800 nm). Since ALA-derived photosensitizers absorb most of the visible spectrum, results from these studies give a good indication of the photoprotective capability of these fabrics throughout this region. Mice were irradiated dorsally with a Kodak slide projector equipped with a 300 W tungsten-halogen lamp (I0 = 48.3 mW/cm2). After determining the minimal phototoxic dose (MPD) to be 1.40 +/- 0.4 J/cm2, we irradiated the tape-stripped ALA-sensitized mice through the stretched test fabrics with appropriate multiples of the MPD. The special fabric provided protection against 25-30 MPD visible light between 360-800 nm in 14/14 mice. The typical fabric failed to provide protection against 2.5 MPD of the same spectrum. No phototoxic or other adverse responses were seen with either the ALA or light control. In conclusion, the Solumbra fabric is much more protective against ALA photosensitization than the typical fabric. Both appear better at blocking UV than visible light.

Aminolevulinic Acid↗

Gout in systemic lupus erythematosus and overlap syndrome - a hospital-based study.

The negative association between gout and rheumatoid arthritis is widely accepted, and gout is also speculated to be rare in systemic lupus erythematosus (SLE), as only a few sporadic cases have been reported. From 1985 to 2001 we encountered 15 lupus patients at Chang-Gung Memorial Hospital, including two with lupus-scleroderma and one with lupus-scleroderma-polymyositis overlap syndrome coexisting with gout. This study retrospectively analyses the clinical and laboratory characteristics of these patients. A lower female predominance is found, and most patients developed gout after the onset of SLE, although gout preceded SLE in two cases. Measurement of serum uric acid and 24-h urine uric acid found all of the patients to be hyperuricaemic and underexcretors of uric acid. Furthermore, most of the patients (14/15) were receiving diuretics. Also, many had hypertension and serious cardiovascular diseases. Renal impairment during gouty attacks seemed to be a predisposing factor for developing end-stage renal disease. Gouty arthritis usually occurred during relative SLE inactivity, podagra was frequent, and tophi were found in a few patients. Compared with the unselected population of SLE patients, the cases studied here had a higher incidence of chronic arthritis, malar rash, haematologic disorder, photosensitivity, serositis and neurologic disorder. Renal disease in the patients sampled was frequently membranous nephropathy.

Adult↗

Chronic actinic dermatitis. An immunohistochemical study of its T-cell antigenic profile, with comparison to cutaneous T-cell lymphoma.

Chronic actinic dermatitis (CAD) describes a persistent photosensitivity disorder in the absence of continued exposure to photosensitizers; it is characterized by a T-cell infiltrate within the epidermis and dermis. The purpose of this study was to characterize the T-cell infiltrate better immunohistochemically. Serial cryostat sections of fresh-frozen punch biopsy specimens of skin were analyzed in 11 patients with CAD and 3 patients with erythrodermic cutaneous T-cell lymphoma (CTCL). Monoclonal antibodies against the pan T-cell, pan B-cell, and T-cell subsets and the T cell-receptor (TCR) antigens were used. CD8-positive (T-suppressor-cytotoxic) cells were predominant in the epidermis of CAD, while CD4-positive (T-helper) cells were predominant in the epidermis and dermis of CTCL. CDw29-positive (T-memory) cells were predominant in all cases. The number of BF1 (beta-chain constant region of the TCR)-positive cells approximated the number of CD3-positive cells in all CAD cases but was significantly lower than the number of CD3-positive cells in two of three cases of CTCL. There was no clustering or preferential staining with any of the beta-chain variable-region antibodies in any of the specimens. These results indicate that CAD has a characteristic immunophenotype distinct from that of most cases of CTCL and that discordance between BF1 and CD3 expressions did not occur in the CAD cases.

Antigens, Differentiation, T-Lymphocyte↗

Photosensitivity: classification, diagnosis, and treatment.

Photosensitivity disorders are common. Phototoxicity due to excess ultraviolet exposure while on a photosensitizing drug and polymorphic light eruption are two frequent examples. Patients should be encouraged to consider Taylor's ABCDEs: Avoidance, Barrier creams, Clothing, Dihydroxyacetone, and Education.

Humans↗

Sunscreens: a review.

The need for daily sunscreen use has become increasingly evident over the last several decades. In a population that spends more recreational time in the sun than ever before, there is a growing awareness of the harmful effects of sunlight exposure. Patients who have a photosensitivity disorder or who are taking photosensitizing medications may also benefit from the use of new broad-spectrum superpotent sunscreens.

Humans↗

Systemic photoprotection with alpha-tocopherol (vitamin E) and beta-carotene.

Cutaneous photodamage is partly mediated via oxidative pathways and there is evidence to suggest that antioxidants within the skin may have a photoprotective effect. Antioxidant activity is provided by a number of naturally occurring substances including alpha-tocopherol (vitamin E) and beta-carotene, whose effects are mediated by their capacity to quench singlet oxygen, scavenge free radicals and prevent the formation of free radicals. Beta-carotene has been used as treatment for various photosensitivity disorders for more than 30 years. The main indication for its use is in the treatment of the photosensitivity associated with erythropoietic protoporphyria. A role for beta-carotene in the prevention of non-melanoma skin cancer has yet to be demonstrated despite clinical research activity in this area. The role for alpha-tocopherol as a photoprotective agent is less clear-cut and it has yet to be established as treatment either for conditions characterized by photosensitivity or as an agent for preventing chronic photodamage or cutaneous malignancy.

Antioxidants↗

Photosensitivity in children: an approach to diagnosis and management.

Photosensitivity disorders in children include a wide array of conditions, many of which are unique to this age group. Prompt diagnosis of these disorders becomes difficult at times because of the overlapping clinical pictures. Genodermatoses and metabolic disorders may have associated systemic involvement, which may lead to these children presenting to pediatricians who may overlook the photosensitivity. A dermatologist's consultation is essential in such cases for specific instructions and counseling of parents regarding photoprotection in these children. This intervention may improve the quality of life by reducing the morbidity and chances of early mortality. This review includes a comprehensive discussion of the distinguishing clinical features of childhood photodermatoses along with general guidelines regarding their investigation and treatment.

Child↗

PUVA therapy for photosensitive psoriasis.

The purpose of this study was to assess the prophylactic effect of oral photochemotherapy with psoralens and UVA (PUVA) on patients with light-sensitive psoriasis. Of fifteen patients with photosensitive psoriasis, ten with a history of polymorphous light eruption (PMLE) slowly developing into psoriasis were treated with trimethylpsoralen (TMP) and UVA. Five patients with no preceding PMLE reaction were similarly treated; two with 8-methoxypsoralen (8-MOP), two with TMP and one in whom 8-MOP was later changed to TMP. Good to excellent results were obtained in 9/10 of the first category and in 3/5 of the second, giving an overall efficacy of 80%. Preexisting psoriatic lesions did not, however, heal during TMP therapy but did so when treated with 8-MOP. The results confirm, for light-sensitive psoriatics, the efficacy of PUVA in photosensitive disorders.

Female↗

Changes of photosensitivity and action spectrum with time in solar urticaria.

BACKGROUND: Solar urticaria is a rare photosensitivity disorder characterized by the rapid onset of a pruritic, erythematous and urticarial rash following sun-exposure. The action spectrum and degree of photosensitivity have been observed to change over time in several isolated reports. METHODS: Monochromator phototesting was performed on multiple occasions on 12 patients with solar urticaria. RESULTS: Six patients demonstrated normal responses in the initial phototest. They remained normal in five patients upon subsequent testing. Seven patients demonstrated abnormal immediate responses, most frequently at UVA wavelengths. Within some individuals, variations in action spectrum and/or degree of photosensitivity were observed over the years, but not over days. CONCLUSION: Significant changes in action spectrum and degree of photosensitivity may occur over years in solar urticaria. However, changes over shorter periods of time are likely to be minor and not have clinical significance.

Adolescent↗

A case of actinic prurigo showing hypersensitivity of skin fibroblasts to ultraviolet A (UVA).

We here report a patient with actinic prurigo. He had had erythematous papulovesicular eruptions on the sun-exposed sites from fall to early summer for 4 years. The lesions healed leaving atrophic scars. The histology showed epidermal necrosis and dermal dense perivascular lymphohistiocytic infiltration and edema. His minimal erythema doses to ultraviolet B (UVB) and UVA were normal and lowered, respectively. Skin lesions were produced by repeated irradiation with UVA plus UVB, but not with UVA alone. Then he was diagnosed as having actinic prurigo. Skin fibroblasts from the patient were hypersensitive to UVA. We believe that the hypersensitivity relates to the pathomechanisms of the photosensitivity in the case. UVA sensitivity of fibroblasts may be useful for differentiating actinic prurigo, hydroa vacciniforme, and other similar photosensitive disorders.

Adult↗